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Fibrodysplasia Ossificans Treatment & Management

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
Updated: Jun 24, 2016

Medical Care

Fibrodysplasia ossificans progressiva (FOP) should be diagnosed during the neonatal period.[27] Early treatment of fibrodysplasia ossificans progressiva helps avoid the factors of aggravation, slow the progression of the disease, and provide the children with improved quality of life. No effective medical therapy is known for fibrodysplasia ossificans progressiva; bisphosphonates and corticosteroids are only beneficial during the flares. Gene therapy may hold promise in fibrodysplasia ossificans progressiva treatment.

Systemic steroids are sometimes used for acute flare-ups. Iontophoresis with steroids or acetic acid may improve diminished range of motion.

The discovery of the FOP gene reveals a highly conserved target in the transforming growth factor-beta/bone morphogenetic protein signaling pathway and compels therapeutic approaches for the development of small-molecule signal transduction inhibitors for activinlike kinase-2.[28] Effective therapies for fibrodysplasia ossificans progressiva may be based on blocking activinlike kinase-2 or blocking of activin receptor IA/activin–like kinase 2 signaling.[29]


Surgical Care

Patients with fibrodysplasia ossificans, a rare disorder, may require oral surgical and anesthetic procedures to control oral pain. The importance of a minimally invasive surgical technique and appropriate anesthetic management has been stressed.[30]

The experience using general anesthesia has been favorable, with awake nasal fiberoptic intubation evaluated as desirable for airway management.[31]

Contributor Information and Disclosures

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.


Slawomir Majewski, MD Professor and Director, Department of Dermatology and Venereology, Warsaw School of Medicine, Poland

Disclosure: Nothing to disclose.

Sebastian S Majewski 

Disclosure: Nothing to disclose.

Stefania Jablonska, MD Chairman, Professor Emeritus, Department of Dermatology, Warsaw School of Medicine, Poland

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey J Miller, MD Associate Professor of Dermatology, Pennsylvania State University College of Medicine; Staff Dermatologist, Pennsylvania State Milton S Hershey Medical Center

Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology, Association of Professors of Dermatology, North American Hair Research Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Jean Paul Ortonne, MD Chair, Department of Dermatology, Professor, Hospital L'Archet, Nice University, France

Jean Paul Ortonne, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

  1. Feldman G, Li M, Martin S, et al. Fibrodysplasia ossificans progressiva, a heritable disorder of severe heterotopic ossification, maps to human chromosome 4q27-31. Am J Hum Genet. 2000 Jan. 66(1):128-35. [Medline].

  2. de la Pena LS, Billings PC, Fiori JL, Ahn J, Kaplan FS, Shore EM. Fibrodysplasia ossificans progressiva (FOP), a disorder of ectopic osteogenesis, misregulates cell surface expression and trafficking of BMPRIA. J Bone Miner Res. 2005 Jul. 20(7):1168-76. [Medline].

  3. Shore EM, Glaser DL, Gannon FH. Osteogenic induction in hereditary disorders of heterotopic ossification. Clin Orthop Relat Res. 2000 May. 303-16. [Medline].

  4. Shore EM, Xu M, Feldman GJ, et al. A recurrent mutation in the BMP type I receptor ACVR1 causes inherited and sporadic fibrodysplasia ossificans progressiva. Nat Genet. 2006 May. 38(5):525-7. [Medline].

  5. Fontaine K, Semonin O, Legarde JP, Lenoir G, Lucotte G. A new mutation of the noggin gene in a French Fibrodysplasia ossificans progressiva (FOP) family. Genet Couns. 2005. 16(2):149-54. [Medline].

  6. Nakajima M, Haga N, Takikawa K, Manabe N, Nishimura G, Ikegawa S. The ACVR1 617G>A mutation is also recurrent in three Japanese patients with fibrodysplasia ossificans progressiva. J Hum Genet. 2007. 52(5):473-5. [Medline].

  7. Furuya H, Ikezoe K, Wang L, et al. A unique case of fibrodysplasia ossificans progressiva with an ACVR1 mutation, G356D, other than the common mutation (R206H). Am J Med Genet A. 2008 Feb 15. 146A(4):459-63. [Medline].

  8. Kaplan FS, Xu M, Seemann P, et al. Classic and atypical fibrodysplasia ossificans progressiva (FOP) phenotypes are caused by mutations in the bone morphogenetic protein (BMP) type I receptor ACVR1. Hum Mutat. 2009 Mar. 30(3):379-90. [Medline].

  9. Petrie KA, Lee WH, Bullock AN, et al. Novel mutations in ACVR1 result in atypical features in two fibrodysplasia ossificans progressiva patients. PLoS One. 2009. 4(3):e5005. [Medline]. [Full Text].

  10. Nakahara Y, Katagiri T, Ogata N, Haga N. ACVR1 (587T>C) mutation in a variant form of fibrodysplasia ossificans progressiva: second report. Am J Med Genet A. 2014 Jan. 164A(1):220-4. [Medline].

  11. Song GA, Kim HJ, Woo KM, Baek JH, Kim GS, Choi JY, et al. Molecular consequences of the ACVR1 R206H mutation of fibrodysplasia ossificans progressiva. J Biol Chem. 2010 May 12. [Medline].

  12. Herrera-Esparza R, Pacheco-Tovar D, Bollain-Y-Goytia JJ, Torres Del Muro F, Ramírez-Sandoval R, Pacheco-Tovar MG, et al. An Activin Receptor IA/Activin-Like Kinase-2 (R206H) Mutation in Fibrodysplasia Ossificans Progressiva. Case Rep Genet. 2013. 2013:260371. [Medline]. [Full Text].

  13. Kaplan FS, Zasloff MA, Kitterman JA, Shore EM, Hong CC, Rocke DM. Early mortality and cardiorespiratory failure in patients with fibrodysplasia ossificans progressiva. J Bone Joint Surg Am. 2010 Mar. 92(3):686-91. [Medline]. [Full Text].

  14. Lin FY, Lin CH, Shu G, Chen CK. Fibrodysplasia ossificans progressiva: initial presentation with a preosseous lesion of the scalp and its MRI appearance. Skeletal Radiol. 2016 Jul. 45 (7):991-6. [Medline].

  15. Pignolo RJ, Bedford-Gay C, Liljesthröm M, Durbin-Johnson BP, Shore EM, Rocke DM, et al. The Natural History of Flare-Ups in Fibrodysplasia Ossificans Progressiva (FOP): A Comprehensive Global Assessment. J Bone Miner Res. 2016 Mar. 31 (3):650-6. [Medline].

  16. Kitterman JA, Strober JB, Kan L, Rocke DM, Cali A, Peeper J, et al. Neurological symptoms in individuals with fibrodysplasia ossificans progressiva. J Neurol. 2012 Dec. 259(12):2636-43. [Medline]. [Full Text].

  17. Kriegbaum RK, Hillerup S. Fibrodysplasia ossificans progressiva (FOP): Report of a case with extra-articular ankylosis of the mandible. J Craniomaxillofac Surg. 2013 Mar 16. [Medline].

  18. Deirmengian GK, Hebela NM, O'Connell M, Glaser DL, Shore EM, Kaplan FS. Proximal tibial osteochondromas in patients with fibrodysplasia ossificans progressiva. J Bone Joint Surg Am. 2008 Feb. 90(2):366-74. [Medline].

  19. Chichareon V, Arpornmaeklong P, Donsakul N. Fibrodysplasia ossificans progressiva and associated osteochondroma of the coronoid process in a child. Plast Reconstr Surg. 1999 Apr. 103(4):1238-43. [Medline].

  20. Levy CE, Lash AT, Janoff HB, Kaplan FS. Conductive hearing loss in individuals with fibrodysplasia ossificans progressiva. Am J Audiol. 1999 Jun. 8(1):29-33. [Medline].

  21. Aslan G, Celik F, Gorgu M. Unusual ankylosis of the jaw due to fibrodysplasia ossificans progressiva. Ann Plast Surg. 1999 Nov. 43(5):576-8. [Medline].

  22. van der Meij EH, Becking AG, van der Waal I. Fibrodysplasia ossificans progressiva. An unusual cause of restricted mandibular movement. Oral Dis. 2006 Mar. 12(2):204-7. [Medline].

  23. Muthu V, Khaire NS, Varma S. Fibrodysplasia ossificans progressiva-primum non nocere. Clin Rheumatol. 2014 Apr. 33(4):591-2. [Medline].

  24. Jeziorska M, Dabska M, Buraczewski J. [Myositis ossificans (clinico-pathological entity often diagnosed erroneously as malignant tumor)]. Nowotwory. 1980 Apr-Jun. 30(2):183-94. [Medline].

  25. Zaghloul KA, Heuer GG, Guttenberg MD, Shore EM, Kaplan FS, Storm PB. Lumbar puncture and surgical intervention in a child with undiagnosed fibrodysplasia ossificans progressiva. J Neurosurg Pediatr. 2008 Jan. 1(1):91-4. [Medline].

  26. Carvalho DR, Farage L, Martins BJ, Speck-Martins CE. Craniofacial findings in fibrodysplasia ossificans progressiva: computerized tomography evaluation. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2011 Apr. 111(4):499-502. [Medline].

  27. Dzukou T, Barbier C, Spyckerelle C, Labarriere F, Vittu G, Kremp O. [Fibrodysplasia ossificans progressiva in children. The interest of early diagnosis and treatment]. Presse Med. 2005 Mar 12. 34(5):373-7. [Medline].

  28. Kaplan FS, Glaser DL, Pignolo RJ, Shore EM. A new era for fibrodysplasia ossificans progressiva: a druggable target for the second skeleton. Expert Opin Biol Ther. 2007 May. 7(5):705-12. [Medline].

  29. Kaplan FS, Le Merrer M, Glaser DL, et al. Fibrodysplasia ossificans progressiva. Best Pract Res Clin Rheumatol. 2008 Mar. 22(1):191-205. [Medline]. [Full Text].

  30. Wadenya R, Fulcher M, Grunwald T, Nussbaum B, Grunwald Z. A description of two surgical and anesthetic management techniques used for a patient with fibrodysplasia ossificans progressiva. Spec Care Dentist. 2010 May. 30(3):106-9. [Medline].

  31. Kilmartin E, Grunwald Z, Kaplan FS, Nussbaum BL. General anesthesia for dental procedures in patients with fibrodysplasia ossificans progressiva: a review of 42 cases in 30 patients. Anesth Analg. 2014 Feb. 118(2):298-301. [Medline].

  32. Altschuler EL. Consideration of Rituximab for fibrodysplasia ossificans progressiva. Med Hypotheses. 2004. 63(3):407-8. [Medline].

Widespread tumors and indurations mainly in the scapular area, found on radiographic examination to consist of heterotopic bone formation.
Typical hallus valgus deformity.
Proliferation of fibroblasts within the muscle with partial replacement of the muscle fibers.
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